Abstract

FMF is a monogenic autoinflammatory disease with recurring episodes of fever and serositis attacks. FMF is associated with mutations in pyrin. On the other hand mutations in a molecule in the same pathway, cryopyrin, is characterized by inflammatory features involving the inner ear as well. A study has suggested the involvement of cochlea in Behcet disease, which is a polygenic autoinflamatory disease.

Highlights

  • FMF is a monogenic autoinflammatory disease with recurring episodes of fever and serositis attacks

  • Children included to the study were diagnosed as FMF according to previously suggested criteria. 74 children with FMF and 20 controls were enrolled to the study

  • Audiometric examinations were carried out including otoacustic emission testing (OAE) by distortion products (DP) and signal noise ratio (SNR) testing with 1000, 1400, 2000, 2800 and 4000 Hz and audiometric evalution with pure tone average (PTA) measurements and high frequency levels of 8000, 10000, 12500, 16000

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Summary

Introduction

FMF is a monogenic autoinflammatory disease with recurring episodes of fever and serositis attacks. FMF is associated with mutations in pyrin. On the other hand mutations in a molecule in the same pathway, cryopyrin, is characterized by inflammatory features involving the inner ear as well. A study has suggested the involvement of cochlea in Behçet disease, which is a polygenic autoinflamatory disease

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